3. DEFINITION AND INTRODUCTION
• Phthisis bulbi is defined as atrophy, shrinkage, and disorganization of the eye and
intraocular contents
• Clinical condition representing end-stage ocular response to severe eye injury or disease
damage
• Not all eyes rendered sightless by trauma become phthisical
• A mnemonic rule used to describe phthisis bulbi is ''7S" referring to :
Soft - Shrunken - Shapeless - Sightless - Structureless - Squared - Sore.
5. GRADING
Yanoff and Fine proposed the following descriptive grading system based on
clinicopathological features:
• Grade I : Atrophy bulbi without shrinkage
• Grade II : Atrophia bulbi with shrinkage
• Grade III : Atrophia bulbi with shrinkage and disorganization (phthisis bulbi)
• Grade IV : Phthisis bulbi with intraocular ossification
• Grade V : Phthisis bulbi with intraocular calcium deposition
6. ATROPHIA BULBI WITHOUT
SHRINKAGE.
• The size and shape of the eye are maintained despite the atrophy of intraocular tissues.
• The following structures are most sensitive to loss of nutrition
• The lens, which becomes cataractous;
• The retina,which atrophies and becomes separated from RPE by serous fluid
accumulation;
• The aqueous outflow tract, where anterior and posterior synechiae develop
7. ATROPHIA BULBI WITH
SHRINKAGE
• The eye becomes soft because of ciliary body dysfunction and progressive reduction of
IOP.
• The globe becomes smaller and assumes a squared-off configuration as a result of the
influence of the 4 EOM
• The anterior chamber collapses.
• Associated corneal endothelial cell damage initially results in corneal edema, followed by
opacification with degenerative pannus, stromal scarring, and vascularization.
• Most of the remaining internal structures of the eye will be atrophic but recognizable
histologically
8. PHTHISIS BULBI
• The size of the globe shrinks from normal average diam of 23–25 mm to an average diam
of 16–19 mm.
• Most of the ocular contents become disorganized.
• In areas of preserved uvea the RPE proliferates, and nodular drusen may develop.
• In addition, extensive dystrophic calcification of the Bowman layer, lens, retina, and drusen
usually occurs.
• Osseous metaplasia of the RPE with bone formation may be a prominent feature.
• Finally, the sclera becomes markedly thickened, particularly posteriorly
9.
10. PATHOPHYSIOLOGY
• The major factors associated with the pathogenesis of phthisis are ocular hypotony,
deranged blood-ocular barriers, and intraocular inflammation.
• Hypotony causes alteration of oxygenation, nutrition and metabolism,
• Breakdown of the ocular blood barrier of the plasma proteins and release of cytokines,
chemotactic & angiogenetic factors
11. DIAGNOSIS
Physical examination
• Phthisical eyes can usually be easily detected by inspection of the patient’s face
• Diagnosis is simplified due to the unilaterality of the disease
• With asymmetry of the eyeballs and interpalpebral fissures.
12. SIGNS
• Microphthalmos
• Enophthalmos
• Lagophthalmos
• Reduced eyelid fissure
• Strabismus
• Conjunctival chemosis and hyperemia
• Corneoscleral scarring, thickening, vascularization, dystrophic
calcification and shrinkage
• Flattening of the anterior chamber with a narrow to closed
chamber angle
• Hypotony
• Neovascularization of the iris and chamber angle (rubeosis
iridis)
• Intraocular inflammation (uveitis/endophthalmitis)
• Synechia (peripheral/posterior)
• Cyclitic/epiretinal membranes (fibrous/fibrovascular)
• Cataract formation
• Phacodonesis (anterior displacement of the ciliary body)
• Choroidal or/and retinal detachment
• Epiretinal membranes
• Choroidal/ciliary body, retinal, and optic nerve
degeneration/atrophy
• Intraocular hemorrhages
• Dystrophic calcification and heterotopic ossification
14. MANAGEMENT
• Treatment approach for a phthisic eye is often futile,
• Mainly aims at alleviating ocular pain and at cosmetic rehabilitation of the affected eye
• Not to restore vision.
• It can be removed, a procedure called enucleation of the eye.
• Sometimes, though, it is possible to transplant only parts of the eye, and some vision can
be restored.
Phthisis bulbi is defined as atrophy,
shrinkage, and disorganization
of the eye and intraocular contents. Not all eyes rendered
sightless by trauma become phthisical. If the nutritional status of the eye and near-normal
intraocular pressure (IOP) are maintained during the repair process,
the globe will
remain
clinically stable. However, blind eyes are at high risk for repeated trauma, with cumulative
destructive effects. Slow, progressive functional decompensation may also prevail
Etiology
Phthisis bulbi is an end stage ocular disease which can result from a variety of ocular insults including. Common causes include trauma, surgery, infection, inflammation, malignancy, retinal detachment, and vascular lesion[2]
Traumatic
Open globe injury
Closed globe injury
Chemical injury —alkali burn
Radiation
Infections
Keratitis
Endophthalmitis
Panophthalmitis
Chronic retinal detachment
Postoperative
Anterior segment surgeries (cataract surgery via anterior route and lensectomy, glaucoma surgery, corneal surgeries)
Posterior segment surgeries (vitrectomy for endophthalmitis, retained intraocular foreign bodies, suprachoroidal hemorrhage, silicone oil removal, vitreoretinal surgery for retinal detachment with complex proliferative vitreoretinopathy, after penetrating trauma, combined rhegmatogenous, & tractional retinal detachment in diabetics, retinectomy for intractable glaucoma pneumatic retinopexy, suprachoroidal hemorrhage drainage)
Inflammation
Intermediate uveitis is the most common inflammatory cause of end-stage eye disease. In the advanced stages of intermediate uveitis, cyclitic membranes may form leading to hypotony and phthisis.
JIA & Childhood-onset Behçet disease
Sympathetic ophthalmia
Vogt-Koyanagi-Harada syndrome
Autosomal dominant neovascular inflammatory vitreoretinopathy.
Vascular disorders
Ocular ischemic syndrome
Coat's disease
Retinopathy of prematurity (ROP)
Proliferative diabetic retinopathy (PDR)
Retinal capillary hemangioblastoma
Congenital Causes
Persistent hyperplastic primary vitreous
Peters’ anomaly type 2
Norrie disease
Familial exudative vitreoretinopathy
Incontinentia pigmenti
Osteoporosis-pseudoglioma syndrome (OPPG)
Tumors
Retinoblastoma
Choroidal melanoma
Rare tumors including adenoma of the nonpigmented ciliary epithelium, double pseudo-neoplastic proliferation of the RPE, primary retinal glial tumor with features of myxopapillary ependymoma, osteogenic sarcoma, concurrent benign teratoid medulloepithelioma and pineoblastoma, and conjunctival spindle cell carcinoma.
Medications
Cidofovir
Miscellaneous
Parry–Romberg syndrome
Uveal effusion syndrome
Sickle cell disease
Neurofibromatosis
STAGES OF OCULAR DEGENERATION Many blind eyes pass through several stages of atrophy
and disorganization
before progressing to the end stage of phthisis bulbi:
• Atrophia bulbi without shrinkage. In this initial stage, the size and shape of the eye
are maintained despite the atrophy of intraocular tissues. The following structures
are most sensitive to loss of nutrition: the lens, which becomes cataractous; the retina,
which atrophies and becomes separated from the retinal
pigment epithelium
(RPE) by serous fluid accumulation; and the aqueous outflow tract, where anterior
and posterior synechiae develop.
A B
C
Figure 1-10 Types of multinucleated giant
cells. A, Langhans (circle). Note the peripheral
arrangement
of nuclei. B, Touton giant
cell (circle). Note the central eosinophilic cytoplasm and
annulus of nuclei surrounded by a foamy, lipid-filled
pale outer ring. C, Foreign body giant
cell
(circle). Note the haphazardly arranged nuclei.
CHAPTER 1: Introduction to Part I ● 15
C
A B
*
*
Figure 1-11 Phthisis bulbi. A, Gross photograph
of a whole
globe. Note the squared-off
shape
of the globe (arrow), resulting from hypotony and the force of the 4 rectus muscles on the
sclera. B, Gross photograph
of a phthisical globe that has been opened. Note the irregular
contour, cataractous lens with calcification (asterisk), cyclitic membrane with adherent retina
(arrowheads),
and bone formation (between green arrows). C, Photomicrograph demonstrating
the histopathologic correlation with the gross photograph
shown in part B. In addition,
organized
ciliochoroidal effusions are apparent histologically (blue arrows). (Part A courtesy of
Ralph C. Eagle,
MD; parts B and C courtesy of Robert H. Rosa Jr, MD.)
• Atrophia bulbi with shrinkage. In this stage, the eye becomes soft because
of ciliary
body dysfunction and progressive reduction of IOP. The globe becomes smaller
and assumes a squared-off
configuration as a result of the influence of the 4 rectus
muscles. The anterior chamber collapses. Associated corneal endothelial cell
damage initially results in corneal edema, followed by opacification with degenerative
pannus, stromal scarring, and vascularization. Most of the remaining internal
structures of the eye will
be atrophic but recognizable histologically.
• Phthisis bulbi (Fig 1-11). In this end stage, the size of the globe shrinks from a
normal average diameter
of 23–25 mm to an average diameter
of 16–19 mm. Most
of the ocular contents become disorganized.
In areas of preserved uvea the RPE
proliferates, and nodular drusen may develop. In addition, extensive dystrophic
calcification of the Bowman layer, lens, retina,
and drusen usually occurs. Osseous
metaplasia of the RPE with bone formation may be a prominent feature. Finally,
the
sclera becomes markedly thickened, particularly posteriorly
Figure 1-11 Phthisis bulbi. A, Gross photograph
of a whole
globe. Note the squared-off
shape
of the globe (arrow), resulting from hypotony and the force of the 4 rectus muscles on the
sclera. B, Gross photograph
of a phthisical globe that has been opened. Note the irregular
contour, cataractous lens with calcification (asterisk), cyclitic membrane with adherent retina
(arrowheads),
and bone formation (between green arrows). C, Photomicrograph demonstrating
the histopathologic correlation with the gross photograph
shown in part B. In addition,
organized
ciliochoroidal effusions are apparent histologically (blue arrows).
History
The term phthisis bulbi derives from the Greek word phthiein or phthinein , meaning shrinkage or consuming, and was first used by Galen. Over the last 200 years, the clinical interpretation of phthisis bulbi has often been modified according to the underlying disease and structural changes; a clear distinction from ocular atrophy was often difficult and controversial. Hogan and Zimmerman were the first ones who stated that both terms – atrophy and phthisis bulbi – refer to consecutive stages in the degeneration process of a severely damaged eye. Their descriptive classification system including three different stages – (1) ocular atrophy without shrinkage; (2) with shrinkage; and (3) with shrinkage and disorganization.
General Pathology
Microscopic features include internal disorganization, inflammatory reaction, a reactive proliferation of various cells, calcification, and ossification.The globe is reduced in size (usually <20 mm) with a thickened/folded posterior sclera. Dystrophic calcification is common, and osseous metaplasia sometimes occurs, forming what is called "intraocular bone".[3]
Pathophysiology
The major factors associated with the pathogenesis of phthisis are ocular hypotony, deranged blood-ocular barriers, and intraocular inflammation.[2] Hypotony causes alteration of oxygenation, nutrition and metabolism, while the breakdown of the ocular blood barrier of the plasma proteins and release of cytokines, chemotactic & angiogenetic factors
Differential diagnosis
Although the underlying diseases and the clinical course of phthisis bulbi are quite variable, the end-stage disease is rarely missed because of characteristic clinical features (i.e., small, soft, atrophic eyes), which are often associated with decreased or lost vision. However, clinicians should be aware of any potential disease entity which, if not treated properly, may result in a blind, often painful phthisical eye. Intraocular malignancies (i.e., retinoblastoma, malignant uveal melanoma) should be taken into consideration if the ocular history is limited and an obvious cause for phthisis is missing. In addition, congenital abnormalities like microphthalmos and microcornea should be kept in the differential diagnosis of phthisis bulbi.
Management
General treatment
Treatment approach for a phthisic eye is often futile, and mainly aims at alleviating ocular pain and at cosmetic rehabilitation of the affected eye, not to restore vision. It can be removed, a procedure called enucleation of the eye. Sometimes, though, it is possible to transplant only parts of the eye, and some vision can be restored.
